Comparison of dural grafts and methods of graft fixation in Chiari malformation type I decompression surgery.

Suboccipital decompression with duraplasty is a widely accepted method for treating patients with Chiari malformation type I. However, important details of the duraplasty technique are still controversial. This retrospective study analyzes clinical and radiological outcomes after surgery depending upon the type of graft and methods of graft fixation. Seventy consecutive decompressions with duraplasty were analyzed. Two types of grafts, nonautologous (Non-AutoG; 60.0%) and autologous (AutoG; 40.0%), and two methods of graft fixation, suturing (S; 67.1%) and gluing (G; 32.9%), were used in four different combinations: (Non-AutoG+S: 31.4%; Non-AutoG+G: 28.6%; AutoG+S: 35.7%; AutoG+G: 4.3%) according to surgeon preference. The mean follow-up was 63.4 months. According to gestalt and Chicago Chiari Outcome Scales, satisfactory results were obtained in 72.9% and 78.6% of cases, respectively, in the long term. The outcomes were not related to the kind of graft (p = 0.44), fixation method (p = 0.89) or duraplasty pattern (p = 0.32). Decreased syringomyelia was observed in 88.9% of cases, and no associations with the kind of graft (p = 0.84), fixation method (p = 1) or duraplasty pattern were found (p = 0.96). Pseudomeningocele occurred 5 times more often in the Non-AutoG group than in the AutoG group (52.4% vs. 10.7%; p < 0.05), whereas their formations were not related to the fixation method (p = 0.34). Three cases (12.0%) required reoperation with reduraplasty. Autologous and nonautologous dural grafts can be sutured or glued with similar clinical results; however, the use of nonautologous grafts is linked with a much higher risk of pseudomeningocele formation.

Prevalence and Outcome of Anterior and Middle Cranial Fossae Encephaloceles without Cerebrospinal Fluid Leak or Meningitis.

BACKGROUND: With advances in imaging techniques, encephaloceles, meningoceles, and meningoencephaloceles are occasionally discovered incidentally. These can be located in anterior cranial fossa (ACF), mostly protruding into sphenoid and ethmoid sinuses, or middle cranial fossa (MCF), protruding into the temporal bone. We reviewed a large series of cranial computed tomography and magnetic resonance imaging scans to identify the prevalence of asymptomatic encephaloceles, meningoceles, and meningoencephaloceles and describe their outcome. METHODS: We retrospectively reviewed a database of all magnetic resonance imaging and computed tomography scans done at Weill Cornell Medicine for any reason between 2003 and 2018. Encephaloceles, meningoceles, or meningoencephaloceles were confirmed on 72 scans. Of these, chart reviews were performed to identify incidentally discovered cases with symptoms other than cerebrospinal fluid leak, and chart reviews and phone calls were conducted to determine patient demographics, treatment, and outcome. RESULTS: There were 18 incidental cases for a prevalence of 0.0074%, of which 6 were located in ACF, and 12 were located in MCF. The mean age for ACF cases was 39 ± 15.9 years and for MCF cases was 49.5 ± 19.8 years. There were no leaks in any cases after the encephaloceles were discovered. Eleven of 12 (91.6%) MCF cases were treated conservatively, while 3 of 6 (50%; P = 0.083) ACF cases were treated surgically. CONCLUSIONS: This study showed that encephaloceles, meningoceles, and meningoencephaloceles without cerebrospinal fluid leak or meningitis in MCF were more often conservatively managed with observation only, whereas these entities in ACF were often repaired prophylactically. Incidentally discovered encephaloceles have a relatively benign natural history and do not precipitously leak.

Tendencia de los Defectos de Tubo Neural en el Estado de Hidalgo México del 2013-2018 / Trend of Neural Tube Defects in Hidalgo Mexico from 2013-2018

INTRODUCCIÓN: los Defectos de Tubo Neural (DTN) son defectos congénitos del sistema nervioso central resultado del cierre inadecuado en alguna zona del tubo neural, siendo los más frecuentes Anencefalia y Espina Bífida en sus diferentes variantes. En Latinoamérica y México se encuentran dentro de las principales causas de morbilidad y mortalidad infantil. MATERIAL Y MÉTODOS: Análisis de series de tiempo de casos y defunciones de DTN en Hidalgo del 2013-2018 generado a partir de la base de datos de la Dirección General de Vigilancia Epidemiológica de los Defectos del Tubo Neural y Craneofaciales proporcionada por la Secretaria de Salud del Estado de Hidalgo, se incluyeron 187 casos con DTN que nacieron y radican en el Estado. Se calcularon las tasas de mortalidad infantil específicas por DTN con el objetivo de identificar probables factores que incidan o incrementen dichas tendencias. RESULTADOS: la incidencia de DTN fue de 58.7 en el periodo estudiado, la Anencefalia es el más incidente 45% (84), seguido del Mielomeningocele 33% (62) él cual muestra una incidencia creciente. El 84% de la población estaba afiliada al Seguro Popular. Solo el 7.5% (14) de las madres de los casos consumieron ácido fólico tres meses previos al embarazo y el 55% (103) acudieron a 3 consultas prenatales o menos. CONCLUSIONES: Los DTN requieren de estudio y vigilancia permanente pues representan una causa importante de morbilidad y mortalidad en la infancia que afecta a los individuos que los padecen, sus familias, la sociedad y el sistema de salud, con esto se evitarían resultados negativos

INTRODUCTION: Neural Tube Defects (DTN) are congenital defects of the central nervous system resulting from an inadequate closure in some area of ​​the neural tube, the most frequent being Anencephaly and Spina Bifida in their different variants. In Latin America and Mexico, they are among the main causes of infant morbidity and mortality. MATERIAL AND METHODS: Analysis of time series of cases and deaths of DTN in Hidalgo from 2013-2018 generated from the database of the General Directorate of Epidemiological Surveillance of Neural Tube and Craniofacial Defects directly by the Ministry of Health of the State of Hidalgo, included 187 cases with DTN that were born and reside in the State. DTN specific infant mortality rates will be calculated in order to identify probable factors that influence or increase various trends. RESULTS: the incidence of DTN was 58.7 in the period studied, Anencephaly is the most incident 45% (84), followed by Myelomeningocele 33% (62), which shows an increasing incidence. 84% of the population was affiliated at the Seguro Popular. Only 7.5% (14) of the mothers of the cases consumed folic acid three months before the pregnancy and 55% (103) attended 3 or less prenatal visits. CONCLUSIONS: DTN are problems of study and permanent surveillance, since they represent an important cause of morbidity and mortality in childhood that affect the individuals who suffer, their families, society and the health system, this would avoid negative results

Tisseel® versus Hemopatch® for dural sealing in neurosurgery. A prospective study in a tertiary center.

BACKGROUND: Dural repair is a potential source of complications in neurosurgery. We make a comparison in pseudomeningocele and CSF leak incidence with the sealants Tisseel® and Hemopatch®. METHODS: We collected 147 patients from September 2017 to December 2018 in a prospective observational study. Inclusion criteria were adult patients with an intradural cranial or spinal surgery whose dura was closed with a fibrin sealant. Primary endpoints were the incidence of pseudo meningocele and CSF leak. Secondary endpoints were the surgical-site infection, epidural hematoma, and the influence of previous surgery. RESULTS: In 65 and 82 patients Tisseel® and Hemopatch® were used as sealants respectively. The incidence of CSF leak presented a significant statistical relation with the use of Tisseel® in a univariate and multivariate analysis. Infratentorial surgery presented a higher incidence of pseudomeningocele and CSF leak, but the approach used was not a significant factor in multivariate analysis. Patients who were operated previously had a higher risk present a postoperative complication. CONCLUSIONS: The incidence of pseudomeningocele and CSF leak was higher with Tisseel® compared with Hemopatch® with a statistic significant relation in case of CSF fistulae. The procedure done may be a confusion factor in our study. There was no report of adverse effects or a higher incidence of complications. However, it is recommended to plan randomized trials with larger samples to get stronger evidence.

Specific birth defects in pregnancies of women with diabetes: National Birth Defects Prevention Study, 1997-2011.

BACKGROUND: Diabetes is associated with an increased risk for many birth defects and is likely to have an increasing impact on birth defect prevalence because of the rise in diabetes in the United States in recent decades. One of the first analyses in which specific birth defects were assessed for their relationship with both pregestational and gestational diabetes used data from the initial 6 years of the National Birth Defects Prevention Study. That analysis reported strong associations for pregestational diabetes with several birth defects, but few exposures among some of the less common birth defects led to unstable estimates with wide confidence intervals. Since that analysis, the study continued to collect data for another 8 years, including information on approximately 19,000 additional cases and 6900 additional controls. OBJECTIVE: Our objective was to use data from the National Birth Defects Prevention Study, the largest population-based birth defects case-control study in the United States, to provide updated and more precise estimates of the association between diabetes and birth defects, including some defects not previously assessed. STUDY DESIGN: We analyzed data on deliveries from October 1997 through December 2011. Mothers of case and control infants were interviewed about their health conditions and exposures during pregnancy, including diagnosis of pregestational (type 1 or type 2) diabetes before the index pregnancy or gestational diabetes during the index pregnancy. Using logistic regression, we separately assessed the association between pregestational and gestational diabetes with specific categories of structural birth defects for which there were at least 3 exposed case infants. For birth defect categories for which there were at least 5 exposed case infants, we calculated odds ratios adjusted for maternal body mass index, age, education, race/ethnicity, and study site; for defect categories with 3 or 4 exposed cases, we calculated crude odds ratios. RESULTS: Pregestational diabetes was reported by 0.6% of mothers of control infants (71 of 11,447) and 2.5% of mothers of case infants (775 of 31,007). Gestational diabetes during the index pregnancy was reported by 4.7% of mothers of control infants (536 of 11,447) and 5.3% of mothers of case infants (1,653 of 31,007). Pregestational diabetes was associated with strong, statistically significant odds ratios (range, 2.5-80.2) for 46 of 50 birth defects considered. The largest odds ratio was observed for sacral agenesis (adjusted odds ratio, 80.2; 95% confidence interval, 46.1-139.3). A greater than 10-fold increased risk was also observed for holoprosencephaly (adjusted odds ratio, 13.1; 95% confidence interval, 7.0-24.5), longitudinal limb deficiency (adjusted odds ratio, 10.1; 95% confidence interval, 6.2-16.5), heterotaxy (adjusted odds ratio, 12.3; 95% confidence interval, 7.3-20.5), truncus arteriosus (adjusted odds ratio, 14.9; 95% confidence interval, 7.6-29.3), atrioventricular septal defect (adjusted odds ratio, 10.5; 95% confidence interval, 6.2-17.9), and single ventricle complex (adjusted odds ratio, 14.7; 95% confidence interval, 8.9-24.3). For gestational diabetes, statistically significant odds ratios were fewer (12 of 56) and of smaller magnitude (range, 1.3- 2.1; 0.5 for gastroschisis). CONCLUSION: Pregestational diabetes is associated with a markedly increased risk for many specific births defects. Because glycemic control before pregnancy is associated with a reduced risk for birth defects, ongoing quality care for persons with diabetes is an important opportunity for prevention.

Duraplasty with Cervical Fascia Autograft to Reduce Postoperative Complications of Posterior Fossa Tumor Surgery with Suboccipital Midline Approach.

BACKGROUND: The suboccipital midline approach is common dealing with posterior fossa tumors but has a high risk of postoperative complications, such as pseudomeningocele, cerebrospinal fluid (CSF) leak, and meningitis. Neurosurgeons used various kinds of method to lower its rate. METHODS: A retrospective, single-center review of patients diagnosed with posterior fossa tumor underwent a suboccipital midline approach. Compare the rates of pseudomeningocele, CSF leak, and meningitis between 2 groups (artificial dura mater or cervical fascia autograft). We get the cervical fascia autograft from the superficial layer of deep cervical fascia just above the trapezius. RESULTS: Our retrospective review involved 123 patients matching the inclusion criteria between January 2009 and April 2019. The complication rate of pseudomeningocele, CSF leak and meningitis were 8.9%, 4.9%, and 17.9%, respectively. The presence of pseudomeningocele or CSF leak for group "artificial" was 11 of 75 (14.67%) and for group "autograft" it was 3 of 48 (6.25%). The rate of meningitis for group "artificial" (24.0%, 18 of 75) was significantly higher (P = 0.027) than the one for group "autograft" (8.33%, 4 of 48). Multivariate regression analysis suggested that the age was negatively correlated with postoperative pseudomeningocele or CSF leak (P = 0.006), with meningitis (P < 0.001). Using cervical fascia autograft decreased the rate of meningitis (P = 0.021) while showing no statistically significant clinical impact on pseudomeningocele or CSF leak. CONCLUSIONS: Applying the cervical fascia autograft to reconstruct the dura during posterior fossa surgery is a simple and effective method to reduce the rate of meningitis as compared with artificial dura mater.

Prevalence of Spontaneous Asymptomatic Facial Nerve Canal Meningoceles: A Retrospective Review.

BACKGROUND AND PURPOSE: The prevalence of patent facial nerve canals and meningoceles along the facial nerve course is unknown. This study aimed to assess the frequency of such findings in asymptomatic patients. MATERIALS AND METHODS: A retrospective review was completed of patients with high-resolution MR imaging of the temporal bone whose clinical presentations were unrelated to facial nerve pathology. Facial nerve canals were assessed for the presence of fluid along each segment and meningoceles within either the labyrinthine segment (fluid-filled distention, ≥1.0-mm diameter) or geniculate ganglion fossa (fluid-filled distention, ≥2.0-mm diameter). If a meningocele was noted, images were assessed for signs of CSF leak. RESULTS: Of 204 patients, 36 (17.6%) had fluid in the labyrinthine segment of the facial nerve canal and 40 (19.6%) had fluid in the geniculate ganglion fossa. Five (2.5%) had meningoceles of the geniculate ganglion fossa; no meningoceles of the labyrinthine segment of the canal were observed. No significant difference was observed in the ages of patients with fluid in the labyrinthine segment of the canal or geniculate ganglion compared with those without fluid (P = .177 and P = .896, respectively). Of the patients with a meningocele, one had a partially empty sella and none had imaging evidence of CSF leak or intracranial hypotension. CONCLUSIONS: Fluid within the labyrinthine and geniculate segments of the facial nerve canal is relatively common. Geniculate ganglion meningoceles are also observed, though less frequently. Such findings should be considered of little clinical importance without radiologic evidence of CSF otorrhea, meningitis, or facial nerve palsy.

Descriptive and risk factor analysis of nonsyndromic sacral agenesis: National Birth Defects Prevention Study, 1997-2011.

Sacral agenesis is a rare birth defect characterized by partial or complete absence of the sacrum. We sought to (a) describe case characteristics, (b) estimate birth prevalence, and (c) identify risk factors for nonsyndromic sacral agenesis using data from the National Birth Defects Prevention Study (NBDPS). The NBDPS was a population-based, case-control study involving pregnancies with estimated dates of delivery from October 1997 through December 2011. We estimated birth prevalence using all NBDPS eligible cases. Using self-reported maternal exposure information, we conducted multivariable logistic regression analysis to identify potential risk factors overall and among women without diabetes. The birth prevalence of sacral agenesis was 2.6/100,000 live births. In the multivariable analysis, multifetal pregnancy, pre-existing Type 1 diabetes, and pre-existing Type 2 diabetes were positively and significantly associated with sacral agenesis, albeit estimates were imprecise. Preexisting Type 1 diabetes was the strongest risk factor (adjusted odds ratio = 96.6, 95% confidence interval = 43.5-214.7). Among women without diabetes, periconceptional smoking was positively and significantly associated with sacral agenesis. Our findings underscore the importance of smoking cessation programs among women planning pregnancy and the importance of better understanding the role of glycemic control before and during pregnancy when designing interventions for primary prevention of sacral agenesis.

Awareness of spina bifida among family of affected child. A cross sectional questionnaire.

OBJECTIVES: To evaluate the awareness of spina bifida (SB), risk factors and possible preventive measures among mothers who had been following in our clinic with a spina bifida affected child. Methods: A cross-sectional questionnaire-based study with 38 mothers of SB patients who are following at SB and hydrocephalus clinic at King Fahad University Hospital, Khobar, Eastern Province, Saudi Arabia. RESULTS: Thirty-eight mother were included in this questionnaire. Most of the participants were Saudi (94.7%). Ten out of 38 women (26.3%) had used medications during pregnancy (pain medications and herbal drugs), 4 out of 38 (10.5%) had been exposed to imaging radiation, while 9 (23.7%) had experienced moderate to high grade fever (39-41°C) during pregnancy. Moreover, the majority (86.8%) of these women did not receive folic acid (FA) before pregnancy, and 42.1% of them did not have FA during their first trimester. Only one (2.6%) gave positive family history of SB while, 6 (15.8%) reported having other SB children.  Conclusion: There is a considerably low level of awareness in mothers of SB patients despite prevalence of this anomaly in the Eastern province. This necessitates an effort from health care providers to educate the community about this birth defect entity. Furthermore, genetic counseling should be encouraged especially in those who have a positive familial history for better understanding. Also, larger sample size with randomized controlled trials and larger epidemiological studies should be implemented.

Prevalence of temporal bone tegmen defects among patients with Marfan syndrome.

BACKGROUND: Marfan syndrome (MFS) is a genetic disorder affecting connective tissue. The composition of the dura can change. Consequently, lumbo-sacral dural herniations and cerebrospinal fluid (CSF) leaks are encountered, however, they have yet to been described in the temporal bone. AIMS/OBJECTIVES: To define the prevalence of temporal bone meningocele or encephalocele among patients with MFS. MATERIALS AND METHODS: Reviewed medical records of all adult patients, diagnosed with MFS, who were treated between 1993 and 2018 at a single academic referral institute. Head targeted CT scans were analyzed. The presence of an anterior or lateral skull base defect was recorded. RESULTS: One-hundred and one patients diagnosed with MFS were identified. Twelve of which had suitable CT scans and were enrolled in the study. The median age of patients with defects was 65 years (range 41-71). Five of the twelve patients (41.6%) had tegmen defect. Of the seven defects found, the median size of the defects was 3 mm (range 2-5 mm). All defects were in the temporal bone, none in the anterior skull base. CONCLUSIONS AND SIGNIFICANCE: The prevalence of radiological evidence of a temporal bone defect among patients with MFS is high. This is a new, important, and potentially life-threatening association with the syndrome.

Tailored Strategies to Manage Cerebrospinal Fluid Leaks or Pseudomeningocele After Surgery for Tethered Cord Syndrome.

BACKGROUND: Cerebrospinal fluid (CSF) leaks are a dreaded complication after surgery for tethered cord and are associated with significant patient morbidity. Although many strategies for managing postoperative CSF leaks exist, this problem is still daunting, especially in very young patients. In this study, we compared different management techniques for CSF leaks or significant pseudomeningocele in patients with tethered cord syndrome (TCS). METHODS: We analyzed a cohort of children who underwent surgery for TCS from January 2011 to March 2016 (n = 260) and postoperatively experienced either a CSF leak or significant pseudomeningocele. A subset of patients presented with CSF leak (n = 25). We analyzed patient age, sex, presentation, leak appearance, management, and outcome. The different techniques of management were compared for efficacy and morbidity. RESULTS: The diseases associated with leak formation included lipomyelomeningocele (n = 16), myelocystocele (n = 4), and myelomeningocele (n = 5). Three children also had hydrocephalus. Management techniques included cystoperitoneal shunt (CPS) (n = 15), primary resuturing with local rotation flap of muscle (n = 3), external ventricular drain placement (n = 1), ventriculoperitoneal shunt (n = 3), external ventricular drainage (n = 1), and a combination of techniques (rotation flap with external drain; n = 1). Five patients who underwent primary wound revision experienced a leak and required a secondary intervention, but none of the patients who underwent CPS had any complications. CONCLUSIONS: In carefully selected cases, CPS performed early after CSF leakage is highly successful with low morbidity. The primary closure can be attempted for low-pressure leaks without an associated pseudomeningocele.

Epileptogenic role of occult temporal encephalomeningocele: Case-control study.

OBJECTIVE: The purpose of this case-control study is to evaluate the prevalence of occult temporal encephalomeningocele (OTE) in patients with temporal lobe epilepsy (TLE) of unknown etiology presenting to an epilepsy center, independently from drug sensitivity. METHODS: We studied 95 patients with TLE (51 female, mean age 49.4 ± 17.1 years) and 151 controls (88 female, mean age 54.1 ± 21.0 years) using a 1.5T brain MRI, including balanced steady-state gradient echo sequences, targeted to the temporal lobes. RESULTS: OTE was found in 5.2% of the TLE population (9.5% of drug-resistant TLE) and in none of the controls (p = 0.008). Two patients with OTE and drug-resistant TLE became seizure-free after lesionectomy (follow-up 18-24 months). CONCLUSION: OTE is not a rare finding in unselected patients with TLE of unknown origin, provided that it is carefully searched. The absence of OTE in a large group of nonepileptic controls adds evidence to its epileptogenic role.

Coexistence of meningoencephalocele and hippocampal sclerosis: a new type of dual pathology.

Both temporal lobe meningoencephalocele (TE) and hippocampal sclerosis (HS) are causes of drug-resistant temporal lobe epilepsy. Spontaneous TE constitutes a rare but well-known and increasingly recognised cause of refractory epilepsy. It is well known that HS may be associated with another neocortical lesion (dual pathology). Here we report for the first time a new type of dual pathology; namely, the coexistence of temporal pole meningoencephalocele and HS.

Pseudomeningocele Aspiration and Blood Patch Effectively Treats Positional Headache Associated With Postoperative Lumbosacral Pseudomeningocele.

STUDY DESIGN: Retrospective chart review with limited prospective follow-up survey. OBJECTIVE: To evaluate the efficacy and safety of blood patch injection for the treatment of positional headaches caused by postoperative lumbosacral pseudomeningoceles. SUMMARY OF BACKGROUND DATA: Pseudomeningocele is one of the most common complications after posterior lumbosacral spinal surgery. Common treatments include bedrest, abdominal binder use, subarachnoid lumbar drainage, and surgical re-exploration for durotomy closure. To date, only small case reports support the use of epidural blood patch injection for symptomatic pseudomeningocele treatment. METHODS: A retrospective chart review analyzed the outcomes and complications of 19 consecutive patients who underwent blood patch injection, with and without pseudomeningocele aspiration, for symptomatic postoperative lumbosacral pseudomeningoceles between 2009 and 2015. An attempt was made to survey patients by phone regarding satisfaction. RESULTS: As of last follow-up (average time = 22.3 months), 16 patients (84%) experienced headache resolution after blood patch injection and did not require further treatment of their pseudomeningocele. In addition to symptomatic improvement, 12 of the 16 successful patients had imaging, which demonstrated pseudomeningocele resolution. Persistent pseudomeningoceles were demonstrated on imaging among all three unsuccessful patients. CONCLUSION: Pseudomeningocele aspiration followed by blood patch is an effective treatment for symptomatic postoperative lumbosacral pseudomenigocele. This is a minimally invasive alternative to surgical re-exploration with durotomy closure. Injections are most effective when performed early after pseudomeningocele development. LEVEL OF EVIDENCE: 4.

PATTERNS OF NEURAL TUBE DEFECTS AT TWO TEACHING HOSPITALS IN ADDIS ABABA, ETHIOPIA A THREE YEARS RETROSPECTIVE STUDY.

BACKGROUND: Neural tube defects (NTDs), one of the most common congenital malformations, are potentially preventable cause of perinatal morbidity and mortality. OBJECTIVES: To give baseline description of NTDs and their outcome at two teaching hospitals in Addis Ababa, Ethiopia. MATERIALS AND METHODS: A retrospective cross sectional descriptive study conducted from September 2009 to August 2012. RESULTS: During the study period out of 28,961 deliveries 177 cases of NTDs were identified, giving an overall NTD prevalence of 6.1/1000. Only 12% (21/177) were diagnosed before 28 weeks of gestation. The mean gestational age at diagnosis of NTDs was 33.8 weeks (±5.5). Majority, 93.2% (165/177), had antenatal care (ANC) follow-up. Most, 72% (127/177), were diagnosed by ultrasound before delivery while 28% (50/177) were identified at the time of delivery or expulsion. Majority, 85.3% (151/177), never received folic acid supplementation. Only less than 1% (2/177) of the mothers started taking folic acid supplementation pre-conceptionally. Only a third, 33.3% (59/177), of the fetuses were born alive while only 13.6% (24/177) were discharged alive. Myelomeningocele, identified in 51.4% (91/177), was the commonest NTD in this study. CONCLUSION AND RECOMMENDATIONS: The proportion of NTDs in this study is among the highest globally reported rates. The practice of periconceptional folic acid supplementation is negligible. And although most had ANC follow-up the vast majority of NTDs were diagnosed late in the third trimester. It is, therefore, highly recommended to consider implementing national preventive strategies to reduce the prevalence of NTDs in Ethiopia.

The cephaloceles: A clinical, epidemiological and therapeutic study of 50 cases.

INTRODUCTION: Cephaloceles are congenital malformations characterized by externalization of the meninges and/or brain tissue through a congenital skull bone defect. In developed countries, this malformation is rare but in our developing countries, this pathology remains one of our concerns. OBJECTIVE: To describe the epidemiological, clinical and therapeutic aspects of the cephaloceles. MATERIALS AND METHOD: A retrospective study was conducted from 2007 to 2013 on all cases of cephalocele supported in the department of neurosurgery of the Yalgado Ouédraogo University Teaching Hospital of Ouagadougou. The malformations were supplemented by CT scan. All patients were operated. The surgical procedure in cephaloceles of the convexity consisted of a direct approach. Sincipital cephaloceles were operated by transcranial approach or combined approach associating transcranial and transnasal approaches. RESULTS: Fifty patients were gathered during the 7-year period. There were 18 boys and 32 girls. The ages ranged from 1 day to 11 years. The cephaloceles were located on the vault of the skull in 78%; the lesion was sincipital in 22%. The malformation was covered with normal skin in 92%; it was pediculated in 78%. CT scan allowed the classification of cases as meningo-encephaloceles in 31 cases (62%) and meningoceles in 18 cases (36%) and one pure encephalocele. The immediate postoperative course was uneventful in 42 cases (84%); 8 postoperative deaths were recorded. At medium and long term, 4 patients (8%) developed complications of psychomotor deficiency in 3 cases and hydrocephalus in 1 case. The main reasons for the poor prognosis were superinfection, hydrocephalus and/or other brain malformations. CONCLUSION: Cephaloceles remain relatively frequent in our practice. After surgery, mental failure and hydrocephalus can occur impairing the prognosis. The most suitable solution is a policy of prevention with folic acid treatment before and during pregnancy and following up adequate pregnancies.

Repeat resective surgery in complex pediatric refractory epilepsy: lessons learned.

OBJECT Resection can sometimes offer the best chance of meaningful seizure reduction in children with medically intractable epilepsy. However, when surgery fails to achieve the desired outcome, reoperation may be an option. The authors sought to investigate outcomes following resective reoperation in pediatric patients with refractory epilepsy, excluding tumoral epilepsies. Differences in preoperative workup between surgeries are analyzed to identify factors influencing outcomes and complications in this complex group. METHODS Medical records were reviewed for all pediatric patients undergoing a repeat resective surgery for refractory epilepsy at the authors' institution between 2005 and 2012. Tumor and vascular etiologies were excluded. Preoperative evaluation and outcomes were analyzed for each surgery and compared. RESULTS Ten patients met all inclusion criteria. The median age at seizure onset was 4.5 months. Preoperative MRI revealed no lesion in 30%. Nonspecific gliosis and cortical dysplasia were the most common pathologies. The majority of preoperative workups included MRI, video-electroencephalography (EEG), and SISCOM. Intracranial EEG was performed for 60% for the first presurgical evaluation and 70% for the second evaluation. The goal of surgery was palliative in 4 patients with widespread cortical dysplasia. The final Engel outcome was Class I in 50%. The rate of favorable outcome (Engel Class I-II) was 70%. The complication rate for the initial surgery was 10%. However, the rate increased to 50% with the second surgery, and 3 of these 5 complications were pseudomeningoceles requiring shunt placement (2 of the 3 patients underwent hemispherotomy). CONCLUSIONS Resective reoperation for pediatric refractory epilepsy has a high rate of favorable outcome and should be considered in appropriate candidates, even as a palliative measure. Intracranial EEG monitoring should be considered on initial workup in cases where the results of imaging or EEG studies are ambiguous or conflicting. Epilepsy secondary to cortical dysplasia, especially if the dysplasia is not seen clearly on MRI, can be difficult to cure surgically. Therefore, in these cases, as large a resection as can be safely accomplished should be done, particularly when the goal is palliative. The rate of complications, particularly pseudomeningocele ultimately requiring shunt placement, is much higher following reoperation, and patients should be counseled accordingly.

Neural tube dysraphism: review of cutaneous markers and imaging.

Neural tube dysraphisms are congenital anomalies resulting from impaired formation of structures along the craniospinal axis during central nervous system development. When these malformations are large or lack a skin covering, they are easily recognized, whereas smaller or skin-covered malformations may not be readily apparent. Due to the intimate embryologic origin of the skin and nervous system, these occult malformations are often heralded by associated cutaneous abnormalities. In this article, the common clinical presentations and cutaneous markers of craniospinal dysraphism are reviewed, along with the recommended imaging modalities.